X 连锁青年性视网膜劈裂症患者难治性闭角型青光眼治疗模式的转变。
A paradigm shift in the treatment of refractory angle closure glaucoma in a patient with X-linked juvenile retinoschisis.
机构信息
VST Center for Glaucoma Care, L V Prasad Eye Institute, Hyderabad, India.
Anant Bajaj Retina Institute, L V Prasad Eye Institute, Hyderabad, India.
出版信息
Ophthalmic Genet. 2023 Dec;44(6):610-617. doi: 10.1080/13816810.2023.2188225. Epub 2023 Mar 16.
BACKGROUND
X-linked retinoschisis (XLRS) is a rare inherited bilateral retinal degeneration caused by mutations in RS1 gene, occurring exclusively in men. Various ocular complications associated with XLRS are reported, and angle closure glaucoma in these eyes is one such complication that is refractory and needs surgery for intraocular pressure control. Glaucoma surgery in these eyes often results in refractory malignant glaucoma with its serious sequelae. Several surgical modifications to prevent this complication have been tried with no or limited success.
METHODOLOGY
In this report, we present a case of XLRS in a young male with a 22-year follow-up. We have described the natural history and progression of retinal disease and glaucoma.
RESULTS
Refractory angle closure glaucoma in our patient was treated with core vitrectomy, phacoemulsification with intraocular lens implantation, and irido-zonulo-hyaloido-vitrectomy. This helped in successful deepening of anterior chamber, good IOP control, and preventing malignant glaucoma.
CONCLUSION
Our case highlights the role of vitrectomy in managing the secondary angle closure glaucoma in eyes with X-LRS.
背景
X 连锁性视网膜劈裂症（XLRS）是一种由 RS1 基因突变引起的罕见遗传性双侧视网膜变性，仅发生于男性。XLRS 相关的各种眼部并发症已有报道，这些眼中的闭角型青光眼就是其中一种难以控制且需要手术治疗的并发症。这些眼中的青光眼手术常导致难治性恶性青光眼及其严重的后遗症。为预防这种并发症，已经尝试了多种手术改良方法，但效果不佳或有限。
方法
在本报告中，我们介绍了一例年轻男性的 XLRS 病例，随访时间为 22 年。我们描述了视网膜疾病和青光眼的自然病史和进展。
结果
我们的患者难治性闭角型青光眼采用核心玻璃体切除术、超声乳化白内障吸除术联合人工晶状体植入术和虹膜-睫状体-脉络膜玻璃体切除术治疗。这有助于成功加深前房，控制良好的眼压，并预防恶性青光眼。
结论
我们的病例强调了玻璃体切除术在治疗 X-LRS 眼中继发性闭角型青光眼的作用。
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